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Response to: ‘Serum complement factor C5a in IgG4-related disease’ by Fukui et al
  1. Hisanori Umehara1,
  2. Mitsuhiro Kawano2
  1. 1 Division of RA and Autoimmune Diseases, Nagahama City Hospital, Shiga, Japan
  2. 2 Division of Rheumatology, Department of Internal Medicine, Graduate School of Medical Science, Kanazawa University, Ishikawa, Japan
  1. Correspondence to Professor Hisanori Umehara, Division of RA and Autoimmune Diseases, Nagahama City Hospital, Shiga 526-8580, Japan; umehara606{at}

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We appreciate the interest shown by Dr S Fukui and colleagues1 in our recent paper, ‘How to diagnose IgG4-related disease (IgG4-RD)’.2

IgG4-RD is characterised by increased serum IgG4 concentrations and number of IgG4-positive plasma cells in affected lesions.3 However, its diagnosis can be difficult, because clinical signs vary depending on the organs affected.

A Japanese team established comprehensive diagnostic criteria for IgG4-RD, 2011 focusing on the concentration of serum IgG4 (>135 mg/dL), numbers of IgG4-positive cells …

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